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Key points

• Orphan diseases are those that are not widely researched: they need not be rare. Likewise, not all rare diseases are orphans.
• Rare lung diseases may involve the lung only or multiple organs.
• Patients’ associations are often a lifeline for people with rare lung diseases, providing a sense of community and advocating for better treatment.
• Governments have provided incentives for pharmaceutical companies to develop treatments for rare and orphan diseases; however, drug prices are often high.
• Doctors lack experience of many rare lung diseases, but all respiratory physicians should ensure they have some clinical and scientific knowledge of the field and are open-minded about diagnosis.

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Chapters

• Editors' introduction
• Foreword from the World Health Organization
• Foreword from the European Commission
• Promoting respiratory health in Europe
• The burden of lung disease
  • Introduction: the global perspective
  • Key points
  • Respiratory deaths in Europe
  • Figure 1
  • Figure 2
  • The impact on health services
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • The “iceberg of disease”
  • Figure 7
  • Recent trends
  • Figure 8
  • Figure 9
  • Figure 10
  • Future projections
  • Terminology and data
  • Data sources
• The economic burden of lung disease
  • Background
  • Key points
  • Methods
  • The cost of respiratory disease
  • Figure 1
  • Discussion
  • Further reading
• Genetic susceptibility
  • Introduction
  • Key points
  • Figure 1
  • Figure 2
  • Monogenic diseases
  • Complex diseases
  • Figure 3
  • Complex traits
  • Conclusions and future prospects
  • Further reading
• Early-life events
  • Introduction
  • Key points
  • Abnormal antenatal lung growth
  • Low birthweight
  • Prematurity and BPD
  • Figure 1
  • Tobacco smoke exposure
  • Viral infections in infancy
  • Wheezy bronchitis
  • Conclusion
  • Further reading
• Diet and nutrition
  • Introduction
  • Key points
  • Diet as a risk factor
  • Nutritional status and interventions
  • Clinical recommendations
  • Further reading
• Outdoor environment
  • Introduction
  • Key points
  • Pollution: composition/sources
  • Pathophysiological effects of air pollutants
  • Respiratory health effects
  • Long-term consequences
  • Figure 1
  • Respiratory disease in adults and children
  • Lung cancer
  • Figure 2
  • The importance of susceptibility
  • Relevance and outlook
  • Further Reading
• Occupational risk factors
  • Introduction
  • Key points
  • Figure 1
  • Exposure history and assessment
  • Latency between exposure and disease
  • Figure 2
  • Maximum workplace concentrations
  • Exposures and their effects
  • Further reading
• Passive smoking
  • Introduction
  • Key points
  • Harmful effects of SHS on health
  • Figure 1
  • Estimates of burden of disease due to SHS
  • Measurement of SHS
  • Health benefits of smoke-free legislation
  • Protection from exposure to SHS
  • Further reading
• Tobacco smoking
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Toxic effects of tobacco smoke
  • Disorders induced by tobacco smoking
  • Figure 2
  • Societal costs of smoking
  • Dependence on tobacco
  • Legislation and prevention of smoking
  • Figure 3
  • Pharmacotherapy for smoking cessation
  • Counselling for smoking cessation
  • Benefits of quitting smoking
  • Cost-effectiveness of smoking cessation
  • ERS guidelines on smoking cessation
  • Future developments
  • Conclusions
  • Further reading
• Indoor environment
  • Introduction
  • Key points
  • Indoor pollutants and health effects
  • Figure 1
  • Figure 2
  • Specific pollutants
  • Figure 3
  • Indoor air quality in schools
  • Figure 4
  • Interventions
  • Conclusion
  • Further reading
• Childhood asthma
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Causes/pathogenesis
  • Clinical manifestations and consequences
  • Prevention
  • Management
  • Prognosis
  • Future developments
  • Research needs
  • Further reading
• Adult asthma
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Causes/pathogenesis
  • Clinical manifestations
  • Figure 2
  • Figure 3
  • Prevention
  • Management
  • Prognosis
  • Future developments and research needs
  • Further reading
• Chronic obstructive pulmonary disease
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Figure 2
  • Figure 3
  • Causes/pathogenesis
  • Clinical manifestations
  • Prevention
  • Management
  • Prognosis
  • Future developments
  • Research needs
  • Further reading
• Cystic fibrosis
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Figure 2
  • Causes/pathogenesis
  • Clinical manifestations and consequences
  • Prevention
  • Management
  • Prognosis
  • Figure 3
  • Future developments
  • Research needs and unanswered questions
  • Further reading
• Bronchiectasis
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Causes/pathogenesis
  • Figure 2
  • Clinical manifestations and consequences
  • Prevention
  • Management
  • Prognosis
  • Future developments
  • Research needs
  • Further reading
• Paediatric respiratory diseases
  • Introduction
  • Key Points
  • Figure 1
  • Acute bronchiolitis
  • Figure 2
  • Figure 3
  • Perinatal respiratory diseases and BPD
  • Figure 4
  • Figure 5
  • Severe community-acquired pneumonia in children
  • Figure 6
  • Tuberculosis in children
  • Figure 7
  • Immunisation
  • Figure 8
  • Congenital respiratory disorders
  • General remarks
  • Further reading
• Tuberculosis
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Causes/pathogenesis
  • Clinical manifestations and consequences
  • Prevention and control
  • Management
  • Prognosis
  • Research needs
  • Future developments
  • Further reading
• Acute lower respiratory infections
  • Introduction
  • Key points
  • Figure 1
  • Influenza
  • Figure 2
  • Pneumonia
  • Figure 3
  • Figure 4
  • Figure 5a
  • Figure 5b
  • Figure 6
  • Prevention
  • Future developments
  • Further reading
• Lung cancer
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Figure 2
  • Figure 3
  • Causes
  • Prevention
  • Management
  • Prognosis
  • Figure 4
  • Future developments
  • Research needs
  • Further reading
• Acute respiratory distress syndrome
  • Introduction
  • Key points
  • Definition and diagnosis
  • Pathogenesis
  • Incidence and outcome
  • Treatment options
  • Future developments
  • Further reading
• Pulmonary vascular disease
  • Introduction
  • Key points
  • Figure 1
  • Figure 2
  • Pulmonary embolism
  • Figure 3
  • Figure 4
  • Pulmonary hypertension
  • Further reading
• Interstitial lung diseases
  • Introduction
  • Key points
  • Epidemiology
  • Figure 1
  • Figure 2
  • Causes/pathogenesis
  • Clinical manifestations and consequences
  • Prevention
  • Management
  • Prognosis
  • Financial burden
  • Figure 3
  • Figure 4
  • Research needs
  • Further reading
• Sleep breathing disorders
  • Introduction
  • Key points
  • Epidemiology
  • The pathophysiology of OSAS
  • Figure 1
  • Clinical manifestations and consequences
  • Prevention
  • Management
  • Figure 2
  • Figure 3
  • Prognosis
  • Obesity hypoventilation syndrome
  • Future developments and research needs
  • Further reading
• Occupational lung diseases
  • Introduction
  • Key points
  • Acute inhalation injuries
  • Occupational infections
  • Asthma
  • COPD
  • Interstitial lung diseases
  • Figure 1
  • Types of ILD
  • Lung cancer
  • Pleural disease
  • Figure 2
  • Figure 3
  • Prevention
  • Conclusion
  • Further reading
• Rare and orphan lung diseases
  • Introduction
  • Key points
  • Specific diseases
  • Diagnosis and support
  • Therapy
  • Future developments
  • Further reading
• Immunisation against respiratory diseases
  • Introduction
  • Key points
  • Influenza
  • Figure 1
  • Pertussis
  • Figure 2
  • Streptococcus pneumoniae
  • Figure 3
  • Tuberculosis
  • Figure 4
  • Developments and research needs
  • Further reading
• Principles of respiratory investigation
  • Introduction
  • Key points
  • History
  • Physical examination
  • Laboratory methods
  • Respiratory function tests
  • Figure 1
  • Imaging techniques
  • Figure 2
  • Figure 3
  • Invasive biopsy techniques
  • Figure 4
  • Further reading
• Principles of respiratory therapy
  • Introduction
  • Key points
  • Pharmacological therapy
  • Figure 1
  • Nonpharmacological therapy
  • Figure 2
  • Figure 3
  • Other forms of treatment
  • Further reading
• Pulmonary rehabilitation
  • Background
  • Key points
  • Definition
  • Rationale
  • Application
  • Outcome
  • Setting and content
  • What is needed?
  • Conclusion
  • Further reading
• Paediatric respiratory medicine
  • Introduction
  • Key points
  • Figure 1
  • Setting
  • Figure 2
  • Training
  • Standardisation of care: towards a common goal
  • Keeping up with developments
  • Conclusion
  • Further reading
• Respiratory intensive care
  • Introduction
  • Key points
  • The role of respiratory medicine in intensive care
  • Figure 1
  • Future prospects
  • Figure 2
  • Further reading
• Thoracic surgery
  • Introduction
  • Key points
  • Standards of care
  • Figure 1
  • Figure 2
  • Figure 3
  • Changing roles
  • The future
  • Further reading
• Medical respiratory specialists
  • Introduction
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
• Allied respiratory professionals
  • Introduction
  • Key points
  • Respiratory technologists and clinical scientists
  • Figure 1
  • Figure 2
  • Respiratory physiotherapists
  • Respiratory nurses
  • Further reading
• Primary care practitioners
  • Introduction
  • Key points
  • Primary care practitioners
  • Primary care and chronic lung disease
  • A role for primary care practitioners
  • Conclusion
  • Further reading
• Specialist clinical training (HERMES)
  • Specialist training in respiratory medicine
  • Key points
  • Figure 1
  • Development strategy for educational standards
  • Figure 2
  • The HERMES projects: adult respiratory medicine
  • Figure 3
  • Figure 4
  • The other HERMES projects
  • Conclusion
  • Further reading
• ERS School and postgraduate education
  • Introduction
  • Key points
  • The HERMES projects
  • The ERS School web platform
  • Figure 1
  • ERS School Courses
  • School publications
  • Educational research seminars
  • Skills-based, hands-on learning
  • Strategic developments in the School
  • Further reading
• Respiratory research
  • Introduction
  • Key points
  • Fields of research
  • Advances in respiratory biomedicine
  • Conclusion
  • Further reading
• Respiratory journals and publications
  • Introduction
  • Key points
  • Figure 1
  • General medical journals
  • Types of journal article
  • Peer review in respiratory journals
  • Assessing quality and status of respiratory journals
  • Publication ethics
  • Advances in publishing
  • Open access
  • Publications of the ERS
  • Conclusions
  • Further reading
• Patient organisations and the European Lung Foundation
  • Introduction
  • Key points
  • The role of patient organisations
  • Figure 1
  • Pan-European patient organisations
  • Respiratory diseases
  • Figure 2
  • Challenges
  • The future
  • Further reading
• Recommendations and policy
  • Introduction
  • Key points
  • Approach
  • Policy context
  • Noncommunicable diseases
  • Tobacco
  • Air quality
  • Tuberculosis
  • Conclusion
  • Further reading